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Clinical Vignette
Published online: 2020-03-26
Submitted: 2020-03-03
Accepted: 2020-03-15
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CORTICOTROPINOMA AS THE UNDERLYING CAUSE OF INTERMITTENT CUSHING’S SYNDROME IN A PATIENT PREVIOUSLY DIAGNOSED WITH PRIMARY PIGMENTED NODULAR ADRENOCORTICAL DISEASE

Agnieszka Łebek-Szatańska, Maria Stelmachowska-Banaś, Grzegorz Zieliński, Andrzej Styk, Karolina M. Nowak, Lucyna Papierska
DOI: 10.5603/EP.a2020.0015
·
Pubmed: 32293705

open access

Ahead of print
Clinical Vignette
Published online: 2020-03-26
Submitted: 2020-03-03
Accepted: 2020-03-15

Abstract

We are presenting the difficulties we encountered with the female patient with previously diagnosed primary pigmented nodular adrenal disease (PPNAD) and conflicting hormonal and radiological findings a few years later. The patient underwent unilateral adrenalectomy in 2010. Despite repeated search, no other components of Carney’s complex have ever been discovered. 8 years later, she was diagnosed with ACTH-dependant Cushing's syndrome. Pituitary MRI showed a lesion of less than 3 mm on the left side of adenohypophysis (suggesting microadenoma) and bilateral inferior petrosal sinus sampling (BIPSS) confirmed this suspicion. The patient underwent effective transsphenoidal adenomectomy and is now eucortisolaemic. The genetic testing for PRKAR1A mutations was negative and the second histopathological evaluation raised some considerable doubts whether the diagnosis of PPNAD was stated correctly.

Abstract

We are presenting the difficulties we encountered with the female patient with previously diagnosed primary pigmented nodular adrenal disease (PPNAD) and conflicting hormonal and radiological findings a few years later. The patient underwent unilateral adrenalectomy in 2010. Despite repeated search, no other components of Carney’s complex have ever been discovered. 8 years later, she was diagnosed with ACTH-dependant Cushing's syndrome. Pituitary MRI showed a lesion of less than 3 mm on the left side of adenohypophysis (suggesting microadenoma) and bilateral inferior petrosal sinus sampling (BIPSS) confirmed this suspicion. The patient underwent effective transsphenoidal adenomectomy and is now eucortisolaemic. The genetic testing for PRKAR1A mutations was negative and the second histopathological evaluation raised some considerable doubts whether the diagnosis of PPNAD was stated correctly.

Get Citation

Keywords

Cushing' s syndrome, Carney complex, primary pigmented nodular adrenocortical disease

About this article
Title

CORTICOTROPINOMA AS THE UNDERLYING CAUSE OF INTERMITTENT CUSHING’S SYNDROME IN A PATIENT PREVIOUSLY DIAGNOSED WITH PRIMARY PIGMENTED NODULAR ADRENOCORTICAL DISEASE

Journal

Endokrynologia Polska

Issue

Ahead of print

Published online

2020-03-26

DOI

10.5603/EP.a2020.0015

Pubmed

32293705

Keywords

Cushing's syndrome
Carney complex
primary pigmented nodular adrenocortical disease

Authors

Agnieszka Łebek-Szatańska
Maria Stelmachowska-Banaś
Grzegorz Zieliński
Andrzej Styk
Karolina M. Nowak
Lucyna Papierska

References (4)
  1. Meinardi JR, Wolffenbuttel BHR, Dullaart RPF. Cyclic Cushing's syndrome: a clinical challenge. Eur J Endocrinol. 2007; 157(3): 245–254.
  2. Kiefer FW, Winhofer Y, Iacovazzo D, et al. mutation causing pituitary-dependent Cushing disease in a patient with Carney complex. Eur J Endocrinol. 2017; 177(2): K7–KK12.
  3. Hernández-Ramírez LC, Tatsi C, Lodish MB, et al. Corticotropinoma as a Component of Carney Complex. J Endocr Soc. 2017; 1(7): 918–925.
  4. Sarlis NJ, Chrousos GP, Doppman JL, et al. Primary pigmented nodular adrenocortical disease: reevaluation of a patient with carney complex 27 years after unilateral adrenalectomy. J Clin Endocrinol Metab. 1997; 82(4): 1274–1278.

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